Multicentric Malignant Peripheral Nerve Sheath Tumors of Small Intestine and Thigh: Report of a Case

Abstract

Primary malignant peripheral nerve sheath tumor (MPNST) of the small bowel is an extremely rare disease. We report a multicentric MPNSTs of the left thigh and jejunum in a 59-year-old male, who visited our hospital twice in seven months because of a subcutaneous lump on the posterior aspect of the left thigh and life-threatening melena respectively. Laparotomy was performed for the small bowel tumor and a segment of the jejunum containing the tumor near its midportion was resected with end-to-end jejunojejunostomy. Histologically, the tumor which had been excised seven months previously was diagnosed as classical MPNST occurring in the left thigh, and that resected this time, as epithelioid MPNST occurring in the jejunum. Distant metastasis to multiple organs occurred one month after resection of the jejunal MPNST, and the patient died three months later. This is the first case of an MPNST arising in the small intestine reported in Taiwan.